The clinical approach to desmoplastic small round cell tumor.

Overview

BACKGROUND AND OBJECTIVES: Desmoplastic small round cell tumor (DSRCT) is an increasingly recognized entity with a historically poor prognosis. This article describes the present clinical management of these patients. METHODS: We retrospectively reviewed our experience with 40 histologically proven cases of desmoplastic small round cell tumor diagnosed between 7/1/72 and 10/1/99. Thirty-five of these patients are the subjects of a previous report. Demographic data, mode of presentation, completeness of resection, and overall survival were assessed. RESULTS: The overall survival from DSRCT remains very poor and in our series was 29% at three years from diagnosis. There is a significant correlation between use of intense alkylator therapy (P6 protocol), and gross total resection with improved overall survival. It is often technically feasible to remove large peritoneal masses using a tangential dissection technique. Extensive hepatic parenchymal or hilar involvement or extensive infiltration of the diaphragm in the region of the septum transversum and hepatic veins usually makes gross total resection impossible with a corresponding worsened prognosis. CONCLUSION: We recommend induction chemotherapy (P6) followed by aggressive surgical debulking and external beam radiotherapy for the treatment of DSRCT. Further progress will require new forms of treatment.